临床儿科杂志 ›› 2019, Vol. 37 ›› Issue (3): 188-.doi: 10.3969/j.issn.1000-3606.2019.03.007

• 综合报道 • 上一篇    下一篇

17 例儿童血栓性微血管病临床分析

林甜甜,陈朝英   

  1. 首都儿科研究所附属儿童医院肾内科(北京 100020)
  • 出版日期:2019-03-15 发布日期:2019-03-25
  • 通讯作者: 陈朝英 电子信箱:chenchaoying484@aliyun.com

Clinical analysis of thrombotic microangiopathy in 17 children

LIN Tiantian, CHEN Chaoying   

  1. Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics, Beijing 100020, China)
  • Online:2019-03-15 Published:2019-03-25

摘要: 目的 探讨儿童血栓性微血管病(TMA)的临床特点、诊疗及预后。方法 回顾分析2008年7月—2017年12 月收治的17例TMA患儿的临床资料。结果 8例男性患儿和9例女性患儿,平均年龄(4.5±1.8)岁,均有溶血性贫血、 血小板减少和急性肾功能不全;17例患儿中经过血浆治疗、透析、激素及免疫抑制剂等综合治疗,10例痊愈或好转出院, 2 例自行外院治疗, 2例放弃治疗, 3例死亡。结论 TMA是一组以微血管性溶血性贫血、血小板减少、微循环中血小板血栓 造成的器官受累为主要表现的急性临床综合征,早期诊断,及个体化的综合治疗可改善预后。

关键词: 血栓性微血管病; 溶血尿毒综合征; 儿童

Abstract: Objective To explore the clinical characteristics, diagnosis, treatment and prognosis of thrombotic microangiopathy (TMA) in children. Method The clinical data of 17 TMA children admitted from July 2008 to December 2017 were retrospectively analyzed. Results Eight male and nine female children with an average age being 4.5±1.8 years had hemolytic anemia, thrombocytopenia and acute renal insufficiency,. All the 17 children were treated with plasma therapy, dialysis, hormones and immunosuppressive agents. Ten children were cured or discharged after improvement. Two children were treated in other hospitals and 2 gave up treatment and 3 died. Conclusion TMA is an acute clinical syndrome characterized by microvascular hemolytic anemia, thrombocytopenia and organ involvement caused by platelet thrombosis in microcirculation. Early diagnosis and individualized comprehensive treatment can improve the prognosis.

Key words:  thrombotic microangiopathy; hemolytic uremic syndrome; child